Cor triatriatum: Presentation, diagnosis and long-term surgical results

Background. Cor triatriatum sinister is a rare, surgically correctable, congenital cardiac anomaly. Fewer than 250 cases have been reported. It can occur as an isolated defect (classic) or in association with other congenital cardiac anomalies (atypical). Methods. A retrospective review of 28 consecutive patients diagnosed at the Royal Children's Hospital in Melbourne, Australia during a 22-year span from 1981 to 2003. Results. There were 13 men (46%) and 15 women (54%). Fifteen patients were less than 1 year of age (neonates, [n=7], 25%; infants, [n=8], 29%). 17 patients (61%) had a communication between the right atrium and either the proximal or distal chamber. Fifteen patients (54%) had atypical cor triatriatum. Median age at presentation was 6 months (range, 0.6 to 240). Twenty-four patients (86%) had presented by 5 years of age. Five patients (18%) underwent emergency surgery. Median age at operation was 7 months (range, I to 243). Twenty-seven patients (96%) underwent preoperative transthoracic echocardiography. Nine patients (32%), all with atypical cor triatriatum, required cardiac catheterization for diagnosis. Twenty-seven patients (96%) were correctly diagnosed before treatment. Twenty-seven patients (96%) were treated surgically. The defect was approached through the right atrium in 26 patients (93%). There was 1 early death and 1 patient died 10 years after repair. Follow-up was 86% complete. At a median follow-up of 98 months (range, 0.2 to 284), all patients including those with atypical cor triatriatum were in New York Heart Association's function class 1. Post-repair survival was 96% and 88% at 5 and 15 years, respectively. Conclusions. Surgery offers good early and long-term results for both classic and atypical cor triatriatum.